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Table 2 Nuclear genes involved in other mitochondrial functions causing Leigh and Leigh-like syndrome

From: Molecular basis of Leigh syndrome: a current look

Mitochondrial function

Associated nuclear genes

Mitochondrial DNA maintenance

FBXL4, POLG, POLG2, SUCLA2, SUCLG1, TWNK, SLC25A4, MPV17

Mitochondrial translation

GTPBP3, TRMU, EARS2, FARS2, IARS2, GFM1, GFM2, LRPPRC,TACO1, MTFMT, C12Orf65, MRPS34, MRPS39 (PTCD3), NARS2, TSFM

Mitochondrial dynamics

SLC25A46, DNM1L, MFN2, RRM2B

Mitochondrial material import/export

SLC25A19

Membrane phosphocomponents

SERAC1

Sulfur dioxygenase

ETHE1

AAA oligomeric +/− ATPase

CLPB, SPG7

RNA import

PNPT1

RNA-specific adenosine deaminase

ADAR, RNASEH1

Nuclear translocation system

RANBP2

Nuclear pore complex

NUP62

Manganese transportation

SLC39A8